Irene T Ma1, Yesenia Rojas2, Prakash M Masand3, Eumenia C Castro4, Ryan W Himes5, Eugene S Kim2, John A Goss6, Jed G Nuchtern2, Milton J Finegold4, Patrick A Thompson7, Sanjeev A Vasudevan8. 1. Department of Surgery, Mayo Clinic Arizona, Phoenix, Arizona. 2. Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas. 3. Department of Radiology, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas. 4. Department of Pathology, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas. 5. Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas. 6. Division of Abdominal Transplantation, Michael E. DeBakey Department of Surgery, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas. 7. Division of Hematology/Oncology, Department of Pediatrics, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas. 8. Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas. Electronic address: sanjeevv@bcm.edu.
Abstract
BACKGROUND: Focal nodular hyperplasia (FNH) is uncommonly diagnosed in pediatric patients and may be difficult to distinguish from a malignancy. We present a review of all children with a tissue diagnosis of FNH at our institution, describe the diagnostic modalities, and provide recommendations for diagnosis and follow-up based on our experience and review of the literature. METHODS: A retrospective review of children <18years of age diagnosed with FNH at a single institution was performed from 2000 to 2013. RESULTS: Twelve patients were identified with a tissue diagnosis of FNH. Two patients required surgical resection of their lesion owing to concern for malignancy. Ten patients were managed expectantly with imaging surveillance after biopsy confirmed a diagnosis of FNH. All patients who underwent MRI had very typical findings including hypointensity on T1 weighted sequences, hyperintensity on T2, and homogenous uptake of contrast on the arterial phase. On follow-up all patients had either a stable lesion or reduction in size. CONCLUSIONS: Focal nodular hyperplasia presents typically in children with liver disease, have undergone chemotherapy, and adolescent females. Young children, particularly <5years of age, without underlying liver disease or history of chemotherapy can pose a diagnostic dilemma. In this unique subgroup of children with FNH, MRI and/or needle biopsy should be adequate diagnostic modalities for these lesions.
BACKGROUND: Focal nodular hyperplasia (FNH) is uncommonly diagnosed in pediatric patients and may be difficult to distinguish from a malignancy. We present a review of all children with a tissue diagnosis of FNH at our institution, describe the diagnostic modalities, and provide recommendations for diagnosis and follow-up based on our experience and review of the literature. METHODS: A retrospective review of children <18years of age diagnosed with FNH at a single institution was performed from 2000 to 2013. RESULTS: Twelve patients were identified with a tissue diagnosis of FNH. Two patients required surgical resection of their lesion owing to concern for malignancy. Ten patients were managed expectantly with imaging surveillance after biopsy confirmed a diagnosis of FNH. All patients who underwent MRI had very typical findings including hypointensity on T1 weighted sequences, hyperintensity on T2, and homogenous uptake of contrast on the arterial phase. On follow-up all patients had either a stable lesion or reduction in size. CONCLUSIONS: Focal nodular hyperplasia presents typically in children with liver disease, have undergone chemotherapy, and adolescent females. Young children, particularly <5years of age, without underlying liver disease or history of chemotherapy can pose a diagnostic dilemma. In this unique subgroup of children with FNH, MRI and/or needle biopsy should be adequate diagnostic modalities for these lesions.
Authors: Andrew M Fleming; Caitlyn Duffy; Jessica Gartrell; M Beth McCarville; Max R Langham; Robert E Ruiz; Teresa Santiago; Andrew J Murphy Journal: J Surg Case Rep Date: 2022-05-27